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ORPHA:398971

Clear cell adenocarcinoma of the ovary

high confidenceDisorder

Also known as: Ovarian clear cell adenocarcinoma

Publications

753

79.5th percentile

Trials

39

Interventional, condition-specific

Researchers

1,215

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

A rare, malignant, epithelilal ovarian neoplasm, composed of clear, eosinophilic and hobnail cells displaying variable degrees of tubulocystic, papillary and solid histological patterns, macroscopically appearing as a typically unilateral mass in the ovary which ranges from solid to cystic. Patients are often diagnosed in early stages and usually present with pelvic pain and pressure, an abdominal mass and/or gastrointestinal problems, such as early satiety or bloating. Association with Lynch syndrome has been reported.

How rare: 1-9 / 1 000 000 — roughly one to nine people per million. In a city the size of Kolkata, perhaps a few dozen.

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

Additional Mondo synonyms (3)

clear cell adenocarcinoma of ovary · ovarian clear cell adenocarcinoma · ovary clear cell adenocarcinoma

Research stages

Trial readiness signals

Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”

2/6 stages with a signal

An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    753 matched papers (360 in last 10 years) Source

  3. Phenotype characterisedNot found

    No HPO disease–phenotype associations via Monarch for these Mondo IDs

  4. Animal modelNot found

    No Alliance genotype “model of” associations via Monarch for these Mondo IDs

  5. Orphan designationNot found

    No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source

  6. Interventional trialPresent

    39 matched on ClinicalTrials.gov (9 recruiting in sample)

Biology

Genes and phenotypes

Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.

Do we know what causes it?

Not yet — the cause hasn't been pinned down in GenCC.

No strong gene–disease assertion joined for this Orphanet entity.

Phenotypes (Monarch / HPO)

None returned for this Mondo ID. That often means “not linked under this ID,” not “no clinical features.”

Animal models (Monarch / Alliance)

None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.

Monarch fetch 2026-07-29

Therapies

Designations, candidates, and chemicals

FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.

Orphan designation (FDA · EMA)

No designation matched this disease via UMLS or preferred name on the FDA OOPD mirror or EMA orphan register. Absence here is not proof that none exists under another wording.

Open Targets candidates

No drugs or clinical candidates returned for this Mondo ID on Open Targets.

CTD chemicals (MyDisease.info)

No CTD chemical associations returned for this Mondo ID.

Literature

Is anyone studying this?

753

753 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.

753 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).

360 in the last 10 years · high confidence · 79.5th percentile (publications denominator)

Phrase hits: 753 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,215

Distinct author names in 200 sampled papers — named people below.

Who's working on it?

People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.

  1. 01
    Wang Y7 papers · 2026

    Ludwig Center for Cancer Genetics and Therapeutics, Johns Hopkins University School of Medicine, Baltimore, MD 21287.

    Papers in Europe PMC
  2. 02
    Aoki D5 papers · 2021

    Department of Obstetrics and Gynecology, School of Medicine, Keio University, Tokyo, Japan. aoki@sc.itc.keio.ac.jp

    Papers in Europe PMC
  3. 03
    Li Y5 papers · 2024

    Department of Clinical Oncology, The First Affiliated Hospital of Xi'an Jiaotong University College of Medicine, Xi'an, Shaanxi 710061, P.R. China.

    Papers in Europe PMC
  4. 04
    Choi CH4 papers · 2025

    Department of Obstetrics and Gynecology, Samsung Medical Center, Seoul, Republic of Korea.

    Papers in Europe PMC
  5. 05
    Mikami M4 papers · 2025

    Department of Obstetrics and Gynecology, Tokai University School of Medicine, Isehara, Kanagawa 259-1193, Japan.

    Papers in Europe PMC
  6. 06
    Tamada Y4 papers · 2021

    Laboratory of Cancer Biology and Molecular Immunology, Graduate School of Pharmaceutical Sciences, The University of Tokyo, Tokyo 113-0033, Japan.

    Papers in Europe PMC
  7. 07
    Xu Y4 papers · 2025

    Department of Breast Surgery, the First Hospital of China Medical University, Shenyang, 110001, Liaoning, China. xuyingying@cmu.edu.cn.

    Papers in Europe PMC
  8. 08
    Zhang C4 papers · 2024

    Department of Obstetrics and Gynecology, Fengxian Hospital Affiliated to the Southern Medical University, Shanghai, China.

    Papers in Europe PMC
  9. 09
    Zhang Y4 papers · 2026

    Gynecological Oncology Research and Engineering Center of Hunan Province, 86 Xiangya Road, Changsha, Hunan, 410018, China. xyzhangyu@csu.edu.cn.

    Papers in Europe PMC
  10. 10
    Aoki Y3 papers · 2016

    Pharmaceutical Research Department 2, Research Division, Chugai Pharmaceutical Co., Ltd., Kamakura, Japan.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

39

interventional trials for this specific condition

39 interventional trials matched this specific condition name; 9 currently recruiting in our sample. 40 trials are registered for clear cell adenocarcinoma, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 11 September 2026

39 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 96.7th percentile).

high confidence · 96.7th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

39 interventional trials matched after quoted-phrase search and title/condition post-filter.

Broader category: clear cell adenocarcinoma

40

Interventional trials for the parent category, exclusive of NCT IDs already counted above. Eligibility for this subtype is not guaranteed.

Worth raising with a clinician. How we count trials.

Recruiting under the broader category

Observational and natural-history studies

2 observational studies match this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.

Recruiting or not-yet-recruiting

Open the complete matched search on ClinicalTrials.gov

Other registries (secondary)

Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.

raw 1 · after dedupe 1 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 1 · dropped 0 · fetched 2026-07-30

Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri

No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).

Uncertain / not reviewed (1)

Where to find support

Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.

Orphanet entry for Clear cell adenocarcinoma of the ovary — check Associations / patient organisations on that page.

India — NPRD

Last verified 2026-07-26

This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.

Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.

How we counted this

Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.

"Clear cell adenocarcinoma of the ovary" OR "Clear cell adenocarcinoma of ovary" OR "Ovarian clear cell adenocarcinoma" OR "ovary clear cell adenocarcinoma"

Run this search on Europe PMC

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Clear cell adenocarcinoma of the ovary" OR "Clear cell adenocarcinoma of ovary" OR "Ovarian clear cell adenocarcinoma" OR "ovary clear cell adenocarcinoma"

Interventional trials matched via: phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).

Study-type breakdown: 39 interventional · 2 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"clear cell adenocarcinoma"

Query health: ok — strategies attempted: phrase; with hits: phrase

Run this search on ClinicalTrials.gov

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • No synonyms dropped by stoplist
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-27T15:22:02.751Z